Prognostic factors predicting overall survival in Moroccan patients with myelodysplastic syndrome.

BACKGROUND: Prognostic scores are an indispensable tool in the management of myelodysplastic syndromes. AIM: Identify prognostic factors influencing overall survival of patients with myelodysplastic syndromes. METHODS: We included all patients with myelodysplastic syndromes treated in the clinical hematology department of the military hospital of Rabat (Morocco). RESULTS: Seventy-four patients were included. The sex ratio [M / F] was 1.8. The median age was 65.5 years. The median follow-up was 45 months. The karyotype prognostic was good in 74%. In univariate analysis, prognostic factors influencing overall survival are: Age (p = 0.024), karyotype (p = 0.033), rate of bone marrow blasts (p = 0.002), transfusion dependence (p = 0.002), number of cytopenias (p = 0.001), hemoglobin (p = 0.009) and neutrophil count (p = 0.003). In multivariate analysis the only prognostic factor that influenced overall survival was the karyotype (p = 0.036). Transfusion dependence (p = 0.072) and the rate of marrow blasts (p = 0.107) was close to significance. The WHO Prognostic Scoring System (WPSS), the International Prognostic Scoring System (IPSS) and the Revised International Prognostic Scoring System (RIPSS) influence overall survival with a statistical significance (p <0.001). CONCLUSION: Our study confirms the prognostic value of the karyotype and validated the WPSS, the IPSS and the RIPSS scores as significant predictors of overall survival in Moroccan patients with myelodysplastic syndromes.

Primary lymphoma of the cranial vault: case report and a systematic review of the literature.

BACKGROUND: Bone involvement is a common finding in many types of lymphoma. Cranial vault involvement is extremely rare, and the majority of patients are found at staging to have concurrent disease in lymph nodes. Thirty-eight cases of primary lymphoma of the cranial vault have been reported to date. METHODS: This article presents a rare case of primary cranial vault lymphoma and conducts a systematic review of the current literature. A total of 36 articles comprising 38 cases were included for analysis. The relevant demographic, clinical, and imaging characteristics, as well as the treatment and outcomes of this unique disease presentation were studied. RESULTS: The average patient age was 60 years. There was no significant difference in patient gender. The predominant patient complaint was a subcutaneous scalp mass. Of the patients, 11.7% were immunocompromised. CT scans showed signs of osteolysis in 74% of lesions and hyperostosis in 5%, and the cranial vault was observed as normal in 18% of cases. MRI was performed in 23 cases. There was a wide range of histological subtypes, with a slight predominance of diffuse large B-cell lymphoma. Treatment consisted of surgery alone, surgery followed by radiotherapy, and surgery followed by radiotherapy and chemotherapy. The follow-up periods ranged from 5 months to 6 years, with a median value of 7 months. Sixteen patients were followed up to 12 months; 13 of them were alive at 1 year from diagnosis. CONCLUSION: Primary cranial vault lymphoma is an extremely rare finding. It should be considered in the differential diagnosis of scalp masses. Although the analysis of outcome of the reported cases is difficult because of the small number of occurrences of this entity and the variability of follow-up, a combination of surgery, radiotherapy, and chemotherapy seems to offer better outcomes.

[Primary granulocytic sarcoma of the peritoneum: a case report and literature review]. / Sarcome granulocytaire à localisation péritonéale primitive : à propos d'un cas et revue de la littérature.

Granulocytic sarcoma is a rare tumor composed of immature granulocytic cells. Prognosis is poor. The periosteum is preferentially involved. A peritoneum localization is unusual. We report the case of a 20 years old man without particular previous pathologies, which brutally presented an ascitic syndrome in a context of health impairment state. The laparoscopy showes many white nodules on all the peritoneum. The histologic examination of one of these nodules showed granulocytic sarcoma. The blood and bone marrow cell count are without any anomaly. The treatment consisted of a standard acute myeloid leukaemia's chemotherapy with very good evolution. The rarity of peritoneal chloroma causes a diagnostic problem, especially in the absence of hematologic abnormalities. It must be mentioned in the presence of peritoneal nodules even if the blood count and bone marrow are normal.

Splenic marginal zone lymphoma associated with hepatitis B virus infection, remission after viral treatment, and splenectomy: A case report and review of the literature.

We report the case of a patient diagnosed with a splenic marginal zone lymphoma with a simultaneous finding of hepatitis B virus infection, who responded to antiviral treatment and splenectomy. We highlighted this association described in the literature and its possible causal role, as well as the available therapeutic choices.

Bing-Neel syndrome revealing Waldenström's macroglobulinemia: a case report.

INTRODUCTION: Bing-Neel syndrome is a lympho-plasmocytic infiltration of the central nervous system. The Bing-Neel syndrome is a rare entity, which often occurs during the evolution of a Waldenström's macroglobulinemia but can in some cases be the revealing mode of it. OBSERVATION: We report the case of a 56-year-old patient with tumoral form of Bing-Neel syndrome revealing Waldentrom's macroglobulinemia. CONCLUSION: This observation describes a rare entity whose diagnosis and therapeutic management is complex.

Acute Laryngeal Dyspnea as the First Presentation of Mantle Cell Lymphoma: A Case Report and Review of the Literature.

INTRODUCTION: Acute laryngeal dyspnea is a life-threatening emergency, and the causes in adults are most often laryngeal tumors or inflammatory edema. Lymphoma of the larynx and especially the mantle cell type is extremely rare. Case Presentation. We report a case of a 43-year-old woman with no particular pathological history. She presented with progressive dyspnea which has evolved towards an inspiratory bradypnea that worsened until she ultimately required an emergency tracheotomy. Biopsies revealed mantle cell lymphoma. The patient has been staged IVB MIPI 6, and she was treated by immunochemotherapy followed by ASCT. The therapeutic evaluation shows a complete remission, 18 months after, and the patient was always disease free. CONCLUSION: The laryngeal localization of the mantle cell lymphoma is extremely rare; it may present catastrophically with acute airway obstruction. The diagnosis is mostly histological, hence the interest of deep biopsy. Given its rarity, the therapeutic strategy must be discussed case by case in a multidisciplinary consultation meeting.

[Treatment of systemic AL amyloidosis: about 25 cases]. / Traitement des amyloses AL systémiques: à propos de 25 cas.

Primarye systemic AL amyloidosis is a rare hematologic disorder. The majority of the therapeutic guidelines are based on phase II studies or on retrospective comparisons and case series. Our study aimed to describe all the cases of primary AL amyloidosis reported in 2 military hospitals and to make a comparison between standard melphalan-dexamethasone protocol and new agents in first-line treatment of patients with this disease. We conducted a retrospective, descriptive and multicentric study of all patients with AL amyloidosis whose data were collected during the period July 2009-June 2016. Twenty five patients were enrolled in the study (12 patients treated with melphalan-dexamethasone and 13 with bortezomib-based protocol or lenalidomide-based protocol). There was no significant difference in the epidemiological, clinical and prognostic features between the 2 groups. After a median follow up of 40 months, median overall survival was 54 months in the melphalan-dexamethasone-treated group and 60 months in the new therapies-treated group (P = 0.98). Progression-free survival was 18 months in the standard treatment group vs 11 months in the 2nd group (p = 0.08). In our small case series we haven't found a superiority of the new therapies compared to the standard protocol. This result should be confirmed by a true prospective study, mainly because of the cost of these new molecules that are not always accessible, especially in developing countries.

[Acquired amegacaryocytic thrombocytopenic purpura hiding acute myeloid leukemia]. / Un purpura thrombopénique amégacaryocytaire acquis qui cache une leucémie aigue myéloblastique.

Acquired amegakaryocytic thrombocytopenic purpura is a very rare condition characterized by severe thrombocytopenia linked to the reduction or disappearance of megakaryocytes in the bone marrow. It may be primary idiopathic or secondary to many pathological conditions including hematologic disorders. We report the case of a 24-year-old patient admitted for haemorrhagic syndrome caused by immunological thrombocytopenic purpura. The diagnosis was acquired amegakaryocytosis after the failure of corticotherapy and the performance of myelography. The patient was treated with ciclosporin with rapid progression to acute myeloblastic leukemia. The progression of acquired amegakaryocytosis to acute leukemia is reported but it is generally not so rapid and above all it is preceded by myelodysplastic syndrome or medullary aplasia. This study highlights the importance of a close follow-up of these pathologies with a benign-like appearance.

[Prolonged molecular response induced by imatinib in Philadelphia positive acute lymphoblastic leukemia A case report and brief review]. / Rémission moléculaire prolongée induite par l'imatinib dans la leucémie aiguë lymphoblastique avec chromosome de Philadelphie Cas clinique et revue de la littérature.

Philadelphia or BCR-ABL positive acute lymphoblastic leukemia (PH+ ALL) is the most common and severe of adult ALL. The only potentially curator treatment remains allogeneic hematopoietic stem cells transplantation (SCT) in first complete remission. The use of imatinib has revolutionized the treatment of chronic myeloid leukemia. Its incorporation into PH + ALL protocols also improved the prognosis of this disease giving better complete remission rates compared to chemotherapy alone. The treatment of patients not eligible for SCT remains controversial. Prolonged use of high dose tyrosine kinase inhibitors (TKI) (ie: imatinib at 600 or 800 mg/j) as maintenance therapy seems to be a reasonable approach. We present a case of prolonged molecular remission of PH+ ALL under TKI alone as maintenance therapy.